Abstrakt

Canakinumab (ILARIS^®) in cryopyrin-associated periodic syndrome (CAPS) patients below 2 years of age: Review of four cases and literature.

Dhilleswara Rao Vana

Cryopyrin-Associated Periodic Syndrome (CAPS) is combination of different autoinflammatory hereditary disorders with same genetic basis. CAPS caused mostly due to mutation of NLRP3 gene (nucleotide-binding domain, leucine-rich family (NLR), pyrin domain containing 3), which is an early onset in children. There are many anti-INF agents, anti-IL blockers have been reported in the literature, and continuous efforts are being made to find more effective and safe biological drugs. Canakinumab (ILARIS®) is a biological medicine approved to treat a group of rare, but severe, inherited autoimmune disorders associated with over-secretion of IL-1. It preclinical and clinical examinations, canakinumab has been reported as an effective IL-1β blocker in patients of all ages, but available clinical data in children below two years is limited. In this study, we presented four recently reported cases and reviewed available literature that demonstrated the efficacy and safety profiles of canakinumab in patients with CAPS and other autoimmune and inflammatory disorders.